Objectives:To evaluate clinical features,treatment strategies,and outcomes of pleural empyema for children who were treated at referral pediatric hospitals in 8 provinces of Iran.Methods:In this retrospective,multicen...Objectives:To evaluate clinical features,treatment strategies,and outcomes of pleural empyema for children who were treated at referral pediatric hospitals in 8 provinces of Iran.Methods:In this retrospective,multicenter cross sectional study,we retrospectively retrieved patients'data from 8 teaching hospitals during 2010 and 2017.A questionnaire was applied and filled,and all data were statistically and descriptively analyzed.Results:In total,191 children(109 males and 82 females)were included.Their mean age was 4.95 years and ranged from 11 months to 16 years.The majority of cases(45.1%)were 1-4 years old.Fever(70.3%),cough(65.6%),tachypnea(53.1%),chest pain(14.6%),and abdominal pain(12%)were the most common manifestations at admission.The mean length of admission in hospital was 16.4 d.Consequently,27 patients(14.1%)were admitted into the pediatric intensive unit because of severe illness,and 15 patients(7.9%)died.Logistic regression analysis showed that younger age(less than 12 months)and presence of underlying diseases(such as cardiovascular disease,immune deficiencies,malignancies,and neuro-developmental delay)significantly increased the mortality rate of patients with pleural empyema(P=0.004 and P=0.001,respectively).Conclusions:Pleural empyema children of younger age and with underlying diseases are at higher risks of death.In addition,guidelines for treating pleural empyema should be developed.展开更多
Schwann cell transplantation is considered one of the most promising cell-based therapy to repair injured spinal cord due to its unique growth-promoting and myelin-forming properties.A the Food and Drug Administration...Schwann cell transplantation is considered one of the most promising cell-based therapy to repair injured spinal cord due to its unique growth-promoting and myelin-forming properties.A the Food and Drug Administration-approved Phase I clinical trial has been conducted to evaluate the safety of transplanted human autologous Schwann cells to treat patients with spinal cord injury.A major challenge for Schwann cell transplantation is that grafted Schwann cells are confined within the lesion cavity,and they do not migrate into the host environment due to the inhibitory barrier formed by injury-induced glial scar,thus limiting axonal reentry into the host spinal cord.Here we introduce a combinatorial strategy by suppressing the inhibitory extracellular environment with injection of lentivirus-mediated transfection of chondroitinase ABC gene at the rostral and caudal borders of the lesion site and simultaneously leveraging the repair capacity of transplanted Schwann cells in adult rats following a mid-thoracic contusive spinal cord injury.We report that when the glial scar was degraded by chondroitinase ABC at the rostral and caudal lesion borders,Schwann cells migrated for considerable distances in both rostral and caudal directions.Such Schwann cell migration led to enhanced axonal regrowth,including the serotonergic and dopaminergic axons originating from supraspinal regions,and promoted recovery of locomotor and urinary bladder functions.Importantly,the Schwann cell survival and axonal regrowth persisted up to 6 months after the injury,even when treatment was delayed for 3 months to mimic chronic spinal cord injury.These findings collectively show promising evidence for a combinatorial strategy with chondroitinase ABC and Schwann cells in promoting remodeling and recovery of function following spinal cord injury.展开更多
BACKGROUND 2D-echocardiography(2DE)has been the primary imaging modality in children with Kawasaki disease(KD)to assess coronary arteries.AIM To report the presence and implications of incidental congenital coronary a...BACKGROUND 2D-echocardiography(2DE)has been the primary imaging modality in children with Kawasaki disease(KD)to assess coronary arteries.AIM To report the presence and implications of incidental congenital coronary artery anomalies that had been misinterpreted as coronary artery abnormalities(CAAs)on 2DE.METHODS Records of children diagnosed with KD,who underwent computed tomography coronary angiography(CTCA)at our center between 2013-2023 were reviewed.We identified 3 children with congenital coronary artery anomalies in this cohort on CTCA.Findings of CTCA and 2DE were compared in these 3 children.RESULTS Of the 241 patients with KD who underwent CTCA,3(1.24%)had congenital coronary artery anomalies on CTCA detected incidentally.In all 3 patients,baseline 2DE had identified CAAs.CTCA was then performed for detailed evaluation as per our unit protocol.One(11-year-boy)amongst the 3 patients had complete KD,while the other two(3.3-year-boy;4-month-girl)had incomplete KD.CTCA revealed separate origins of left anterior descending artery and left circumflex from left sinus[misinterpreted as dilated left main coronary artery(LCA)on 2DE],single coronary artery(interpreted as dilated LCA on 2DE)and dilated right coronary artery on 2DE in case of anomalous origin of LCA from the main pulmonary artery.The latter one was subsequently operated upon.CONCLUSION CTCA is essential for detailed assessment of coronary arteries in children with KD especially in cases where there is suspicion of congenital coronary artery anomalies.Relying solely on 2DE may not be sufficient in such cases,and findings from CTCA can significantly impact therapeutic decision-making.展开更多
Objective:To investigate the diagnostic status and electrocardiographic correlates in patients with biochemical evidence of iron overload.Methods:We conducted a retrospective cohort study of patients in our hospital w...Objective:To investigate the diagnostic status and electrocardiographic correlates in patients with biochemical evidence of iron overload.Methods:We conducted a retrospective cohort study of patients in our hospital with ferritin levels exceeding 500 ng/mL between January 1,2011,and October 24,2022(corresponding to the pre-COVID-19 pandemic period in Beijing).Using ICD-10-CM coded medical records,we assessed the following:definitive diagnostic characterization(genetic or acquired),electrocardiographic(ECG)completion rates,and the prevalence of ECG abnormalities.Statistical analyses,encompassing chi-square tests and correlation studies,were performed using SPSS Statistics software(version 27.0).Results:Except for cases of malignancy,infectious diseases,hematological diseases,chronic diseases,for the unexplained diagnosis group found elevated ferritin during annual health checkup,there were 17 cases in the group with ferritin above 1,000 ng/ml and 36 cases in the group with ferritin ranging from 500 to 1,000 ng/ml,accounting for 23.2%and 25.8%of the entire ferritin analysis respectively,and the total proportion in the entire analysis was 24.0%.Among the cases indicating ferritin higher than 500ng/ml,24.0%of the cases were of unknown diagnosis.ECG acquisition rate for was 55.7%,with 24% demonstrating abnormalities,including atrial fibrillation,sinus tachycardia arrhythmia,atrioventricular block,prolonged QT interval,T-wave inversion,and ST-segment depression.Conclusion:The study revealed that the proportion of unexplained diagnoses of ferritin overload remains relatively high,and the analysis of the ECG is also insufficient.There is a need to enhance clinicians’awareness and attention to iron overload in both diagnosis and ECG analysis.展开更多
Efficient siRNA delivery is highly desirable for disease treatment.However,the application of conventional nanoparticles is limited by the inability to escape from endo-lysosomes.Herein,we report a strategy using smal...Efficient siRNA delivery is highly desirable for disease treatment.However,the application of conventional nanoparticles is limited by the inability to escape from endo-lysosomes.Herein,we report a strategy using small-molecule drugs to enhance siRNA endo-lysosomal release,addressing this challenge.We encapsulated gentamicin(GM)into the marketed Onpattro■ formulation to establish LNP-siRNA/GM nanoparticles that promote siRNA endo-lysosomal escape through endosomal disruption,mechanistically exhibiting unique functionality and synergistic effects of LNP-siRNA/GM to improve cancer therapy.Besides,GM induced reactive oxygen species(ROS)and phospholipids accumulation in endolysosomes,as well as the physical characteristics of lipid nanoparticles(LNPs)were preserved.We also revealed that GM causes endo-lysosomal swelling and disrupts the endosomal membrane to enable siRNA release,as confirmed by Galectin 3 recruitment and acridine orange release.This approach achieved∼81%mRNA-EGFR silencing,which is more than LNP-siEGFR(∼56.23%)by enhancing siRNA endo-lysosomal escape efficiency.Meanwhile,LNP-siEGFR/GM exhibited significant biological activities in HepG2 cells,driven by the synergistic effects of siEGFR and GM with the VEGF and CXCL12 downregulation of,and ROS and phospholipids upregulation.Furthermore,tumor growth was notably suppressed after intravenous injection of LNP-siEGFR/GM in tumor-bearing nude mice.The combination of EGFR-siRNA and GM could also greatly inhibit angiogenesis,be antiproliferative,and induce tumor cells apoptosis.Therefore,this GM and siRNA co-delivery system would provide an efficient strategy for siRNA endosomal escape,significantly improving knockdown in various LNPs based siRNA delivery systems and efficiently enhancing cancer therapy.展开更多
BACKGROUND: This study was done to compare the admission Full Outline of Unresponsiveness(FOUR) score and Glasgow Coma Scale(GCS) as predictors of outcome in children with impaired consciousness.METHODS: In this obser...BACKGROUND: This study was done to compare the admission Full Outline of Unresponsiveness(FOUR) score and Glasgow Coma Scale(GCS) as predictors of outcome in children with impaired consciousness.METHODS: In this observational study, children(5–12 years) with impaired consciousness of <7 days were included. Children with traumatic brain injury, on sedatives or neuromuscular blockade; with pre-existing cerebral palsy, mental retardation, degenerative brain disease, vision/hearing impairment; and seizure within last 1 hour were excluded. Primary outcomes: comparison of area under curve(AUC) of receiver operating characteristic(ROC) curve for in-hospital mortality. Secondary outcomes: comparison of AUC of ROC curve for mortality and poor outcome on Pediatric Overall Performance Category Scale at 3 months.RESULTS: Of the 63 children, 20 died during hospital stay. AUC for in-hospital mortality for GCS was 0.83(CI 0.7 to 0.9) and FOUR score was 0.8(CI 0.7 to 0.9) [difference between areas –0.0250(95%CI 0.0192 to 0.0692), Z statistic 1.109, P=0.2674]. AUC for mortality at 3 months for GCS was 0.78(CI 0.67 to 0.90) and FOUR score was 0.74(CI 0.62 to 0.87)(P=0.1102) and AUC for poor functional outcome for GCS was 0.82(CI 0.72 to 0.93) and FOUR score was 0.79(CI 0.68 to 0.9)(P=0.2377), which were also comparable. Inter-rater reliability for GCS was 0.96 and for FOUR score 0.98.CONCLUSION: FOUR score was as good as GCS in prediction of in-hospital and 3-month mortality and functional outcome at 3 months. FOUR score had a good inter-rater reliability.展开更多
The incidence of pediatric inflammatory bowel disease (IBD) is rising and recent advances in diagnostics and therapeutics have improved the care provided to these children. There are distinguishing features worth noti...The incidence of pediatric inflammatory bowel disease (IBD) is rising and recent advances in diagnostics and therapeutics have improved the care provided to these children. There are distinguishing features worth noting between early onset and adult onset IBD. Physical and psychosocial development remains a critical target for the comprehensive management of pediatric IBD. Children are not just little adults and consideration must be given to the stages of development and how these stages impact disease presentation and management. The final stage will be the transition from pediatric care to that of adult oriented care and special consideration must be given to make this a successful process. This review highlights special considerations in the management of the child with IBD.展开更多
Fetal arrhythmias reach up around 10%of the total third-level perinatal cardiology references.Sustained bradycardia is defined as a baseline fetal heart rate(FHR)of less than 110 bpm sustained for at least 10 min.The ...Fetal arrhythmias reach up around 10%of the total third-level perinatal cardiology references.Sustained bradycardia is defined as a baseline fetal heart rate(FHR)of less than 110 bpm sustained for at least 10 min.The overall incidence of malignant fetal bradyarrhythmias,such as complete atrioventricular block(AVB)and channellopathies,is relatively rare,1:5000 pregnancies,but represents a serious emergency for the gynecologist,neonatologists,and pediatric cardiologists.Fetal complete AVB is strongly associated with maternal connective tissue disease,but it can be also associated with congenital heart disease and usually with a poorer prognosis with high risk of fetal hydrops and abortion.Currently,the treatment of severe fetal bradyarrhythmias is principally pharmacological and aims to increase the FHR,besides an early resolution of underlying causes,when possible,and a promptly management of fetal heart failure.Intrauterine electrostimulation nowadays is an experimental pioneering method,reserved for limited selected cases.展开更多
BACKGROUND Endocardial fibroelastosis(EFE)is a diffuse endocardial collagen and elastin hyperplasia disease of unknown etiology,which may be accompanied by myocardial degenerative changes leading to acute or chronic h...BACKGROUND Endocardial fibroelastosis(EFE)is a diffuse endocardial collagen and elastin hyperplasia disease of unknown etiology,which may be accompanied by myocardial degenerative changes leading to acute or chronic heart failure.However,acute heart failure(AHF)without obvious associated triggers is rare.Prior to the report of endomyocardial biopsy,the diagnosis and treatment of EFE are highly susceptible to being confounded with other primary cardiomyopathies.Here,we report a case of pediatric AHF caused by EFE mimicking dilated cardiomyopathy(DCM),with the aim of providing a valuable reference for clinicians to early identify and diagnose EFE-induced AHF.CASE SUMMARY A 13-mo-old female child was admitted to hospital with retching.Chest X-ray demonstrated enhanced texture in both lungs and an enlarged heart shadow.Color doppler echocardiography showed an enlarged left heart with ventricular wall hypokinesis and decreased left heart function.Abdominal color ultrasonography revealed a markedly enlarged liver.Pending the result of the endomyocardial biopsy report,the child was treated with a variety of resuscitative measures including nasal cannula for oxygen,intramuscular sedation with chlorpromazine and promethazine,cedilanid for cardiac contractility enhancement,and diuretic treatment with furosemide.Subsequently,the child’s endomyocardial biopsy report result was confirmed as EFE.After the above early interventions,the child’s condition gradually stabilized and improved.One week later,the child was discharged.During a 9-mo follow-up period,the child took intermittent low-dose oral digoxin with no signs of recurrence or exacerbation of the heart failure.CONCLUSION Our report suggests that EFE-induced pediatric AHF may present in children over 1 year of age without any apparent precipitants,and that the associated clinical presentations are grossly similar to that of pediatric DCM.Nonetheless,it is still possible to be diagnosed effectively on the basis of the comprehensive analysis of auxiliary inspection findings before the result of the endomyocardial biopsy is reported.展开更多
AIM To investigate the use of a multidisciplinary,longitudinal simulation to educate pediatric residents and nurses on management of pediatric diabetic ketoacidosis.METHODS A multidisciplinary,multiple step simulation...AIM To investigate the use of a multidisciplinary,longitudinal simulation to educate pediatric residents and nurses on management of pediatric diabetic ketoacidosis.METHODS A multidisciplinary,multiple step simulation course was developed by faculty and staff using a modified Delphi method from the Pediatric Simulation Center and pediatric endocrinology department.Effectiveness of the simulation for the residents was measured with a pre-and post-test and a reference group not exposed to simulation.A follow up post-test was completed 3-6 mo after the simulation.Nurses completed a survey regarding the education activity.RESULTS Pediatric and medicine-pediatric residents(n=20)and pediatric nurses(n=25)completed the simulation course.Graduating residents(n=16)were used as reference group.Pretest results were similar in the control and intervention group(74%±10%vs 76%±15%,P=0.658).After completing the intervention,participants improved in the immediate post-test in comparison to themselves and the control group(84%±12%post study;P<0.05).The 3-6 mo follow up post-test results demonstrated knowledge decay when compared to their immediate post-test results(78%±14%,P=0.761).Residents and nurses felt the interdisciplinary and longitudinal nature of the simulation helped with learning.CONCLUSION Results suggest a multidisciplinary,longitudinal simulation improves immediate post-intervention knowledge but important knowledge decay occurs,future studies are needed to determine ways to decrease this decay.展开更多
Objective Vasopressin has showed a beneficial use in pediatric patients with vasodilatory shock after cardiac surgery. However, the optimal timing of vasopressin initiation has not been investigated.Our aim was to eva...Objective Vasopressin has showed a beneficial use in pediatric patients with vasodilatory shock after cardiac surgery. However, the optimal timing of vasopressin initiation has not been investigated.Our aim was to evaluate the effect of early vasopressin initiation for these patients.展开更多
Rationale:Hepatitis C in the pediatric population is a large health burden globally.With its diverse genotypes as well as genotypic subtypes,there is a discrepancy in the genotypes used in research compared to their p...Rationale:Hepatitis C in the pediatric population is a large health burden globally.With its diverse genotypes as well as genotypic subtypes,there is a discrepancy in the genotypes used in research compared to their prevalence.HCV genotype 6 which is endemic to South China and Southeast Asia comprises approximately one-third of all HCV infections worldwide,but make up a minority of cases studied in HCV research.Patient concerns:We report a case of HCV-6 seen in an 11-yearold Burmese immigrant to the U.S.and describe the new direct acting antiviral treatment guidelines for pediatrics with HCV genotype 6.Interventions:The patient completed a 12-week course of ledipasvir/sofosbuvir(90 mg/400 mg),per FDA weight-based recommendations for treatment-naive HCV genotypes 4-6,without any complications.Outcomes:The patient was treated successfully with an undetectable HCV viral load one month after treatment completion.Lessons:HCV-6,although previously uncommon in the U.S.,is becoming more prevalent.Updated guidelines include the use of direct acting antivirals,which have been proven effective for HCV-6.Lessons on barriers to care in the immigrant population as well as the value of HCV genotyping are also discussed.展开更多
The current research joins previous studies in examining post-traumatic stress disorder (PTSD) and burnout among healthcare workers. The research focuses on the experiences of pediatric residents working in an emergen...The current research joins previous studies in examining post-traumatic stress disorder (PTSD) and burnout among healthcare workers. The research focuses on the experiences of pediatric residents working in an emergency department both in normal times and during the Covid pandemic. Research conducted prior to and during the Covid pandemic outbreak shows negative psychological effects among healthcare workers. Most of that research was conducted within the positivistic-quantitative paradigm. The current study is qualitative and focuses on pediatric residents who provide medical services to a unique population in a peripheral region of Israel, namely the Bedouin-Arab population. The research questions are the following: What characterizes pediatric residents’ work, in general and during the pandemic? Do they show signs of burnout and secondary trauma? How do they perceive their work with the Bedouin-Arab population, especially during the pandemic? The study, conducted within the phenomenological genre, included 14 pediatric residents in a large hospital in Israel’s periphery. Semi-structured clinical interviews were employed, in addition to questionnaires that examined PTSD and burnout to enhance the reliability of the findings. The results show that all residents reported stressful incidents in which patients’ physical integrity was threatened. The residents described the special nature of the medical cases they treated in routine times and during the pandemic outbreak, which stems mostly from the specific characteristics the population of Israel’s periphery. While at the early stages of the pandemic, the residents experienced reduced work pressure, they reported substantial difficulties later in the crisis, which intensified their sense of physical and emotional stress. Most residents reported feeling inadequately prepared for dealing with traumatic events. According to the results, most residents displayed secondary trauma (12 participants in interviews and 11 in questionnaires), which can be classified into categories based on the DSM-5. In the interviews, all 14 participants reported various signs of burnout. The questionnaires indicated burnout symptoms among 10 participants. Giving a voice to pediatric residents, the study highlights the complexity of their routine work as well as their role during the Covid crisis. Based on the findings, recommendations have been made for policymakers. The study highlights the importance of raising awareness to the implications of the residents’ rough work conditions in routine and emergency times and to the need to develop social support and intervention programs that might improve their well-being during their professional work.展开更多
Background:Most children in need of cardiac pacemakers remain dependent on the function of the permanent from childhood to adulthood.We sought to evaluate and compare the function between epicardial and endocardial pa...Background:Most children in need of cardiac pacemakers remain dependent on the function of the permanent from childhood to adulthood.We sought to evaluate and compare the function between epicardial and endocardial pacemakers in pediatric groups with different conditions.Methods:Between 2012 and 2018,this single-canter study evaluated 44 pediatric patients with indications for epicardial or endocardial pacemakers.Results:The 2 groups,at a median age of 5(0.1–16)years,were compared concerning the characteristics of the leads used(n=80:bipolar,unipolar,steroid-eluting,and non–steroid-eluting),survival data,and complications.The reason for pacemaker implantation was congenital complete heart block in 11(25%)cases and postoperative heart block in 33(75%)cases.The commonest congenital heart disease accompanied by postoperative block was the ventricular septal defect.In the endocardial lead group,the mean ventricular pacing threshold immediately after the implantation and during the follow-up was less than that in the epicardial lead group(0.75 vs.0.81 V;P=0.01 and 0.8 vs.2.4 V;P=0.001).During the follow-up,the mean battery longevity was better in the endocardial group(last visit:6.7 endocardial vs.3.3 years epicardial).Lead failure was commoner in the epicardial pacemaker,and chronic high-pacing threshold pattern was seen in 14 patients in this group.After 3 years,freedom from lead failure was 94%and 63%in the endocardial and epicardial leads.Conclusions:Pacemakers with endocardial bipolar steroid-eluting leads showed better lead characteristics regarding survival and battery longevity than epicardial pacemakers without these lead characteristics.An appropriate pacemaker type should be selected based on the patient’s condition.展开更多
Background:The European Congenital Heart Surgeons Association(ECHSA)Congenital Heart Surgery Database(CHSD)was founded in 1999 and is open for worldwide participation.The current dataset includes a large amount of sur...Background:The European Congenital Heart Surgeons Association(ECHSA)Congenital Heart Surgery Database(CHSD)was founded in 1999 and is open for worldwide participation.The current dataset includes a large amount of surgical data from both Europe and China.The purpose of this analysis is to compare patterns of practice and outcomes among pediatric congenital heart defect surgeries in Europe and China using the ECHSA-CHSD.Methods:We examined all European(125 centers,58,261 operations)and Chinese(13 centers,23,920 operations)data in the ECHSA-CHSD from 2006-2018.Operative mortality,postoperative length of stay,median patient age and weight were calculated for the ten benchmark operations for China and Europe,respectively.Results:Benchmark procedure distribution frequencies differed between Europe and China.In China,ventricular septal defect repair comprised approximately 70%of procedures,while Norwood operations comprised less than one percent of all procedures.Neonatal cardiac procedures were rare in China overall.For procedures in STAT mortality category 1,Chinese centers had lower operative mortality rates,while procedures in categories 3 and 5 mortality is lower in European centers.Operative mortality over the time period decreased from 3.89%to 1.64%for the whole cohort,with a sharper decline in China.This drop coincides with an increase of submitted procedures over this 13-year-period.Conclusion:Chinese centers had higher programmatic volume of congenital heart surgeries,while European centers have a more complex case mix.Palliation for patients with functionally univentricular heart was performed less commonly in China.These comparison of patterns of practice and outcomes demonstrate opportunities for continuing bidirectional transcontinental collaboration and quality improvement.展开更多
Neurons are energy-demanding cells.Disruptions in energy metabolism can quickly interrupt neuronal function,leading to cell death and neurodegeneration.For instance,ischemia rapidly depletes adenosine triphosphate(ATP...Neurons are energy-demanding cells.Disruptions in energy metabolism can quickly interrupt neuronal function,leading to cell death and neurodegeneration.For instance,ischemia rapidly depletes adenosine triphosphate(ATP)thereby disrupting energy-dependent cellular processes crucial for homeostasis,and axon degeneration is preceded by a collapse of axonal ATP levels.展开更多
Axon disconnection in the central nervous system(CNS) usually causes signal transduction failure and severe functional deficits in patients with neurological disorders. Currently, there is no cure for patients with CN...Axon disconnection in the central nervous system(CNS) usually causes signal transduction failure and severe functional deficits in patients with neurological disorders. Currently, there is no cure for patients with CNS axon injury and they usually suffer from life-long neurological defects(e.g., paralysis, loss of sensory function, and autonomic dysfunction) and life-threatening complications(e.g., autonomic dysreflexia).展开更多
Implications for lncRNAs in the central nervous system:Transcriptional dysregulation is a key contributor to the pathogenesis of a wide range of diseases and long non-coding RNAs(lncRNAs)are highly expressed in the ne...Implications for lncRNAs in the central nervous system:Transcriptional dysregulation is a key contributor to the pathogenesis of a wide range of diseases and long non-coding RNAs(lncRNAs)are highly expressed in the nervous system.Indeed,amongst the over 50,000 lncRNAs expressed in the human genome,more than 40%are specifically expressed in the brain where their roles in brain development,neuron functions,maintenance.展开更多
Background The associations among genetic variants,cardiac magnetic resonance(CMR)features,and prognostic factors of pediatric heart failure(HF)remain unknown.This study aimed to explore the relationship between genet...Background The associations among genetic variants,cardiac magnetic resonance(CMR)features,and prognostic factors of pediatric heart failure(HF)remain unknown.This study aimed to explore the relationship between genetic differences in pediatric HF and CMR findings and their impact on prognosis of HF in children.Methods This retrospective study included children with a first-time diagnosis of HF.All patients underwent echocardiogram,CMR and genetic testing.The cohort was divided into three subgroups according to late gadolinium enhancement(LGE)on CMR:the no LGE subgroup,the subendocardial LGE subgroup,and the nonsubendocardial subgroup.The endpoint was defined as a lack of improvement in HF on echocardiography after more than six months of follow-up.Results Ninety-five children with HF were included,of whom 64 underwent genetic testing and 89 had echocardiograms at follow-up.There were 20 patients in the no LGE group,31 in the subendocardial group,and 44 in the nonsubendocardial group.The most common genotype was MYH7(27.0%).RBM20 and LMNA both presented a dilated phenotype,whereas GTPBP3 uniformly presented a hypertrophic phenotype.TNNI3 showed no LGE on CMR(P=0.003)and maintained a restrictive phenotype.The subendocardial group was more likely to have septal LGE(93.5%,P<0.001).Multivariable regression analysis indicated that a family history of cardiomyopathy,hydropericardium,and right ventricular ejection fraction(RVEF)were independent predictors of the endpoint event(P=0.035,P=0.005 and P=0.032,respectively).Notably,nonsubendocardial LGE was associated with a worse prognosis than no LGE was(P=0.030).Conclusions In pediatric non-ischemic HF patients,subendocardial LGE can be observed.A family history of cardiomyopathy,hydropericardium,and RVEF were predictors of HF with no improvement.The prognosis of HF is associated with the presence of LGE,particularly nonsubendocardial LGE,and less so with the genetic phenotype.展开更多
基金This study was supported by eight educational hospitals in eight provinces of Iran.
文摘Objectives:To evaluate clinical features,treatment strategies,and outcomes of pleural empyema for children who were treated at referral pediatric hospitals in 8 provinces of Iran.Methods:In this retrospective,multicenter cross sectional study,we retrospectively retrieved patients'data from 8 teaching hospitals during 2010 and 2017.A questionnaire was applied and filled,and all data were statistically and descriptively analyzed.Results:In total,191 children(109 males and 82 females)were included.Their mean age was 4.95 years and ranged from 11 months to 16 years.The majority of cases(45.1%)were 1-4 years old.Fever(70.3%),cough(65.6%),tachypnea(53.1%),chest pain(14.6%),and abdominal pain(12%)were the most common manifestations at admission.The mean length of admission in hospital was 16.4 d.Consequently,27 patients(14.1%)were admitted into the pediatric intensive unit because of severe illness,and 15 patients(7.9%)died.Logistic regression analysis showed that younger age(less than 12 months)and presence of underlying diseases(such as cardiovascular disease,immune deficiencies,malignancies,and neuro-developmental delay)significantly increased the mortality rate of patients with pleural empyema(P=0.004 and P=0.001,respectively).Conclusions:Pleural empyema children of younger age and with underlying diseases are at higher risks of death.In addition,guidelines for treating pleural empyema should be developed.
基金supported in part by NIH R01 NS100531,R01 NS103481NIH R21NS130241(to LD)+3 种基金Merit Review Award I01 BX002356,I01 BX003705 from the U.S.Department of Veterans AffairsIndiana Spinal Cord and Brain Injury Research Foundation(No.19919)Mari Hulman George Endowment Funds(to XMX)Indiana Spinal Cord&Brain Injury Research Fund from ISDH(to NKL and LD)。
文摘Schwann cell transplantation is considered one of the most promising cell-based therapy to repair injured spinal cord due to its unique growth-promoting and myelin-forming properties.A the Food and Drug Administration-approved Phase I clinical trial has been conducted to evaluate the safety of transplanted human autologous Schwann cells to treat patients with spinal cord injury.A major challenge for Schwann cell transplantation is that grafted Schwann cells are confined within the lesion cavity,and they do not migrate into the host environment due to the inhibitory barrier formed by injury-induced glial scar,thus limiting axonal reentry into the host spinal cord.Here we introduce a combinatorial strategy by suppressing the inhibitory extracellular environment with injection of lentivirus-mediated transfection of chondroitinase ABC gene at the rostral and caudal borders of the lesion site and simultaneously leveraging the repair capacity of transplanted Schwann cells in adult rats following a mid-thoracic contusive spinal cord injury.We report that when the glial scar was degraded by chondroitinase ABC at the rostral and caudal lesion borders,Schwann cells migrated for considerable distances in both rostral and caudal directions.Such Schwann cell migration led to enhanced axonal regrowth,including the serotonergic and dopaminergic axons originating from supraspinal regions,and promoted recovery of locomotor and urinary bladder functions.Importantly,the Schwann cell survival and axonal regrowth persisted up to 6 months after the injury,even when treatment was delayed for 3 months to mimic chronic spinal cord injury.These findings collectively show promising evidence for a combinatorial strategy with chondroitinase ABC and Schwann cells in promoting remodeling and recovery of function following spinal cord injury.
文摘BACKGROUND 2D-echocardiography(2DE)has been the primary imaging modality in children with Kawasaki disease(KD)to assess coronary arteries.AIM To report the presence and implications of incidental congenital coronary artery anomalies that had been misinterpreted as coronary artery abnormalities(CAAs)on 2DE.METHODS Records of children diagnosed with KD,who underwent computed tomography coronary angiography(CTCA)at our center between 2013-2023 were reviewed.We identified 3 children with congenital coronary artery anomalies in this cohort on CTCA.Findings of CTCA and 2DE were compared in these 3 children.RESULTS Of the 241 patients with KD who underwent CTCA,3(1.24%)had congenital coronary artery anomalies on CTCA detected incidentally.In all 3 patients,baseline 2DE had identified CAAs.CTCA was then performed for detailed evaluation as per our unit protocol.One(11-year-boy)amongst the 3 patients had complete KD,while the other two(3.3-year-boy;4-month-girl)had incomplete KD.CTCA revealed separate origins of left anterior descending artery and left circumflex from left sinus[misinterpreted as dilated left main coronary artery(LCA)on 2DE],single coronary artery(interpreted as dilated LCA on 2DE)and dilated right coronary artery on 2DE in case of anomalous origin of LCA from the main pulmonary artery.The latter one was subsequently operated upon.CONCLUSION CTCA is essential for detailed assessment of coronary arteries in children with KD especially in cases where there is suspicion of congenital coronary artery anomalies.Relying solely on 2DE may not be sufficient in such cases,and findings from CTCA can significantly impact therapeutic decision-making.
文摘Objective:To investigate the diagnostic status and electrocardiographic correlates in patients with biochemical evidence of iron overload.Methods:We conducted a retrospective cohort study of patients in our hospital with ferritin levels exceeding 500 ng/mL between January 1,2011,and October 24,2022(corresponding to the pre-COVID-19 pandemic period in Beijing).Using ICD-10-CM coded medical records,we assessed the following:definitive diagnostic characterization(genetic or acquired),electrocardiographic(ECG)completion rates,and the prevalence of ECG abnormalities.Statistical analyses,encompassing chi-square tests and correlation studies,were performed using SPSS Statistics software(version 27.0).Results:Except for cases of malignancy,infectious diseases,hematological diseases,chronic diseases,for the unexplained diagnosis group found elevated ferritin during annual health checkup,there were 17 cases in the group with ferritin above 1,000 ng/ml and 36 cases in the group with ferritin ranging from 500 to 1,000 ng/ml,accounting for 23.2%and 25.8%of the entire ferritin analysis respectively,and the total proportion in the entire analysis was 24.0%.Among the cases indicating ferritin higher than 500ng/ml,24.0%of the cases were of unknown diagnosis.ECG acquisition rate for was 55.7%,with 24% demonstrating abnormalities,including atrial fibrillation,sinus tachycardia arrhythmia,atrioventricular block,prolonged QT interval,T-wave inversion,and ST-segment depression.Conclusion:The study revealed that the proportion of unexplained diagnoses of ferritin overload remains relatively high,and the analysis of the ECG is also insufficient.There is a need to enhance clinicians’awareness and attention to iron overload in both diagnosis and ECG analysis.
基金supported by National Natural Science Foundation of China(81502688)Cooperation Research Funding of Capital Medical University(2020KJ000514)+1 种基金Cooperation Research Funding of Capital Medical University(2023KJ000814)R&D Program of Beijing Municipal Education Commission(KM202210025024).
文摘Efficient siRNA delivery is highly desirable for disease treatment.However,the application of conventional nanoparticles is limited by the inability to escape from endo-lysosomes.Herein,we report a strategy using small-molecule drugs to enhance siRNA endo-lysosomal release,addressing this challenge.We encapsulated gentamicin(GM)into the marketed Onpattro■ formulation to establish LNP-siRNA/GM nanoparticles that promote siRNA endo-lysosomal escape through endosomal disruption,mechanistically exhibiting unique functionality and synergistic effects of LNP-siRNA/GM to improve cancer therapy.Besides,GM induced reactive oxygen species(ROS)and phospholipids accumulation in endolysosomes,as well as the physical characteristics of lipid nanoparticles(LNPs)were preserved.We also revealed that GM causes endo-lysosomal swelling and disrupts the endosomal membrane to enable siRNA release,as confirmed by Galectin 3 recruitment and acridine orange release.This approach achieved∼81%mRNA-EGFR silencing,which is more than LNP-siEGFR(∼56.23%)by enhancing siRNA endo-lysosomal escape efficiency.Meanwhile,LNP-siEGFR/GM exhibited significant biological activities in HepG2 cells,driven by the synergistic effects of siEGFR and GM with the VEGF and CXCL12 downregulation of,and ROS and phospholipids upregulation.Furthermore,tumor growth was notably suppressed after intravenous injection of LNP-siEGFR/GM in tumor-bearing nude mice.The combination of EGFR-siRNA and GM could also greatly inhibit angiogenesis,be antiproliferative,and induce tumor cells apoptosis.Therefore,this GM and siRNA co-delivery system would provide an efficient strategy for siRNA endosomal escape,significantly improving knockdown in various LNPs based siRNA delivery systems and efficiently enhancing cancer therapy.
文摘BACKGROUND: This study was done to compare the admission Full Outline of Unresponsiveness(FOUR) score and Glasgow Coma Scale(GCS) as predictors of outcome in children with impaired consciousness.METHODS: In this observational study, children(5–12 years) with impaired consciousness of <7 days were included. Children with traumatic brain injury, on sedatives or neuromuscular blockade; with pre-existing cerebral palsy, mental retardation, degenerative brain disease, vision/hearing impairment; and seizure within last 1 hour were excluded. Primary outcomes: comparison of area under curve(AUC) of receiver operating characteristic(ROC) curve for in-hospital mortality. Secondary outcomes: comparison of AUC of ROC curve for mortality and poor outcome on Pediatric Overall Performance Category Scale at 3 months.RESULTS: Of the 63 children, 20 died during hospital stay. AUC for in-hospital mortality for GCS was 0.83(CI 0.7 to 0.9) and FOUR score was 0.8(CI 0.7 to 0.9) [difference between areas –0.0250(95%CI 0.0192 to 0.0692), Z statistic 1.109, P=0.2674]. AUC for mortality at 3 months for GCS was 0.78(CI 0.67 to 0.90) and FOUR score was 0.74(CI 0.62 to 0.87)(P=0.1102) and AUC for poor functional outcome for GCS was 0.82(CI 0.72 to 0.93) and FOUR score was 0.79(CI 0.68 to 0.9)(P=0.2377), which were also comparable. Inter-rater reliability for GCS was 0.96 and for FOUR score 0.98.CONCLUSION: FOUR score was as good as GCS in prediction of in-hospital and 3-month mortality and functional outcome at 3 months. FOUR score had a good inter-rater reliability.
文摘The incidence of pediatric inflammatory bowel disease (IBD) is rising and recent advances in diagnostics and therapeutics have improved the care provided to these children. There are distinguishing features worth noting between early onset and adult onset IBD. Physical and psychosocial development remains a critical target for the comprehensive management of pediatric IBD. Children are not just little adults and consideration must be given to the stages of development and how these stages impact disease presentation and management. The final stage will be the transition from pediatric care to that of adult oriented care and special consideration must be given to make this a successful process. This review highlights special considerations in the management of the child with IBD.
文摘Fetal arrhythmias reach up around 10%of the total third-level perinatal cardiology references.Sustained bradycardia is defined as a baseline fetal heart rate(FHR)of less than 110 bpm sustained for at least 10 min.The overall incidence of malignant fetal bradyarrhythmias,such as complete atrioventricular block(AVB)and channellopathies,is relatively rare,1:5000 pregnancies,but represents a serious emergency for the gynecologist,neonatologists,and pediatric cardiologists.Fetal complete AVB is strongly associated with maternal connective tissue disease,but it can be also associated with congenital heart disease and usually with a poorer prognosis with high risk of fetal hydrops and abortion.Currently,the treatment of severe fetal bradyarrhythmias is principally pharmacological and aims to increase the FHR,besides an early resolution of underlying causes,when possible,and a promptly management of fetal heart failure.Intrauterine electrostimulation nowadays is an experimental pioneering method,reserved for limited selected cases.
文摘BACKGROUND Endocardial fibroelastosis(EFE)is a diffuse endocardial collagen and elastin hyperplasia disease of unknown etiology,which may be accompanied by myocardial degenerative changes leading to acute or chronic heart failure.However,acute heart failure(AHF)without obvious associated triggers is rare.Prior to the report of endomyocardial biopsy,the diagnosis and treatment of EFE are highly susceptible to being confounded with other primary cardiomyopathies.Here,we report a case of pediatric AHF caused by EFE mimicking dilated cardiomyopathy(DCM),with the aim of providing a valuable reference for clinicians to early identify and diagnose EFE-induced AHF.CASE SUMMARY A 13-mo-old female child was admitted to hospital with retching.Chest X-ray demonstrated enhanced texture in both lungs and an enlarged heart shadow.Color doppler echocardiography showed an enlarged left heart with ventricular wall hypokinesis and decreased left heart function.Abdominal color ultrasonography revealed a markedly enlarged liver.Pending the result of the endomyocardial biopsy report,the child was treated with a variety of resuscitative measures including nasal cannula for oxygen,intramuscular sedation with chlorpromazine and promethazine,cedilanid for cardiac contractility enhancement,and diuretic treatment with furosemide.Subsequently,the child’s endomyocardial biopsy report result was confirmed as EFE.After the above early interventions,the child’s condition gradually stabilized and improved.One week later,the child was discharged.During a 9-mo follow-up period,the child took intermittent low-dose oral digoxin with no signs of recurrence or exacerbation of the heart failure.CONCLUSION Our report suggests that EFE-induced pediatric AHF may present in children over 1 year of age without any apparent precipitants,and that the associated clinical presentations are grossly similar to that of pediatric DCM.Nonetheless,it is still possible to be diagnosed effectively on the basis of the comprehensive analysis of auxiliary inspection findings before the result of the endomyocardial biopsy is reported.
基金Supported by A University of Alabama at Birmingham Department of Pediatrics Founders Fund Grant
文摘AIM To investigate the use of a multidisciplinary,longitudinal simulation to educate pediatric residents and nurses on management of pediatric diabetic ketoacidosis.METHODS A multidisciplinary,multiple step simulation course was developed by faculty and staff using a modified Delphi method from the Pediatric Simulation Center and pediatric endocrinology department.Effectiveness of the simulation for the residents was measured with a pre-and post-test and a reference group not exposed to simulation.A follow up post-test was completed 3-6 mo after the simulation.Nurses completed a survey regarding the education activity.RESULTS Pediatric and medicine-pediatric residents(n=20)and pediatric nurses(n=25)completed the simulation course.Graduating residents(n=16)were used as reference group.Pretest results were similar in the control and intervention group(74%±10%vs 76%±15%,P=0.658).After completing the intervention,participants improved in the immediate post-test in comparison to themselves and the control group(84%±12%post study;P<0.05).The 3-6 mo follow up post-test results demonstrated knowledge decay when compared to their immediate post-test results(78%±14%,P=0.761).Residents and nurses felt the interdisciplinary and longitudinal nature of the simulation helped with learning.CONCLUSION Results suggest a multidisciplinary,longitudinal simulation improves immediate post-intervention knowledge but important knowledge decay occurs,future studies are needed to determine ways to decrease this decay.
文摘Objective Vasopressin has showed a beneficial use in pediatric patients with vasodilatory shock after cardiac surgery. However, the optimal timing of vasopressin initiation has not been investigated.Our aim was to evaluate the effect of early vasopressin initiation for these patients.
文摘Rationale:Hepatitis C in the pediatric population is a large health burden globally.With its diverse genotypes as well as genotypic subtypes,there is a discrepancy in the genotypes used in research compared to their prevalence.HCV genotype 6 which is endemic to South China and Southeast Asia comprises approximately one-third of all HCV infections worldwide,but make up a minority of cases studied in HCV research.Patient concerns:We report a case of HCV-6 seen in an 11-yearold Burmese immigrant to the U.S.and describe the new direct acting antiviral treatment guidelines for pediatrics with HCV genotype 6.Interventions:The patient completed a 12-week course of ledipasvir/sofosbuvir(90 mg/400 mg),per FDA weight-based recommendations for treatment-naive HCV genotypes 4-6,without any complications.Outcomes:The patient was treated successfully with an undetectable HCV viral load one month after treatment completion.Lessons:HCV-6,although previously uncommon in the U.S.,is becoming more prevalent.Updated guidelines include the use of direct acting antivirals,which have been proven effective for HCV-6.Lessons on barriers to care in the immigrant population as well as the value of HCV genotyping are also discussed.
文摘The current research joins previous studies in examining post-traumatic stress disorder (PTSD) and burnout among healthcare workers. The research focuses on the experiences of pediatric residents working in an emergency department both in normal times and during the Covid pandemic. Research conducted prior to and during the Covid pandemic outbreak shows negative psychological effects among healthcare workers. Most of that research was conducted within the positivistic-quantitative paradigm. The current study is qualitative and focuses on pediatric residents who provide medical services to a unique population in a peripheral region of Israel, namely the Bedouin-Arab population. The research questions are the following: What characterizes pediatric residents’ work, in general and during the pandemic? Do they show signs of burnout and secondary trauma? How do they perceive their work with the Bedouin-Arab population, especially during the pandemic? The study, conducted within the phenomenological genre, included 14 pediatric residents in a large hospital in Israel’s periphery. Semi-structured clinical interviews were employed, in addition to questionnaires that examined PTSD and burnout to enhance the reliability of the findings. The results show that all residents reported stressful incidents in which patients’ physical integrity was threatened. The residents described the special nature of the medical cases they treated in routine times and during the pandemic outbreak, which stems mostly from the specific characteristics the population of Israel’s periphery. While at the early stages of the pandemic, the residents experienced reduced work pressure, they reported substantial difficulties later in the crisis, which intensified their sense of physical and emotional stress. Most residents reported feeling inadequately prepared for dealing with traumatic events. According to the results, most residents displayed secondary trauma (12 participants in interviews and 11 in questionnaires), which can be classified into categories based on the DSM-5. In the interviews, all 14 participants reported various signs of burnout. The questionnaires indicated burnout symptoms among 10 participants. Giving a voice to pediatric residents, the study highlights the complexity of their routine work as well as their role during the Covid crisis. Based on the findings, recommendations have been made for policymakers. The study highlights the importance of raising awareness to the implications of the residents’ rough work conditions in routine and emergency times and to the need to develop social support and intervention programs that might improve their well-being during their professional work.
文摘Background:Most children in need of cardiac pacemakers remain dependent on the function of the permanent from childhood to adulthood.We sought to evaluate and compare the function between epicardial and endocardial pacemakers in pediatric groups with different conditions.Methods:Between 2012 and 2018,this single-canter study evaluated 44 pediatric patients with indications for epicardial or endocardial pacemakers.Results:The 2 groups,at a median age of 5(0.1–16)years,were compared concerning the characteristics of the leads used(n=80:bipolar,unipolar,steroid-eluting,and non–steroid-eluting),survival data,and complications.The reason for pacemaker implantation was congenital complete heart block in 11(25%)cases and postoperative heart block in 33(75%)cases.The commonest congenital heart disease accompanied by postoperative block was the ventricular septal defect.In the endocardial lead group,the mean ventricular pacing threshold immediately after the implantation and during the follow-up was less than that in the epicardial lead group(0.75 vs.0.81 V;P=0.01 and 0.8 vs.2.4 V;P=0.001).During the follow-up,the mean battery longevity was better in the endocardial group(last visit:6.7 endocardial vs.3.3 years epicardial).Lead failure was commoner in the epicardial pacemaker,and chronic high-pacing threshold pattern was seen in 14 patients in this group.After 3 years,freedom from lead failure was 94%and 63%in the endocardial and epicardial leads.Conclusions:Pacemakers with endocardial bipolar steroid-eluting leads showed better lead characteristics regarding survival and battery longevity than epicardial pacemakers without these lead characteristics.An appropriate pacemaker type should be selected based on the patient’s condition.
文摘Background:The European Congenital Heart Surgeons Association(ECHSA)Congenital Heart Surgery Database(CHSD)was founded in 1999 and is open for worldwide participation.The current dataset includes a large amount of surgical data from both Europe and China.The purpose of this analysis is to compare patterns of practice and outcomes among pediatric congenital heart defect surgeries in Europe and China using the ECHSA-CHSD.Methods:We examined all European(125 centers,58,261 operations)and Chinese(13 centers,23,920 operations)data in the ECHSA-CHSD from 2006-2018.Operative mortality,postoperative length of stay,median patient age and weight were calculated for the ten benchmark operations for China and Europe,respectively.Results:Benchmark procedure distribution frequencies differed between Europe and China.In China,ventricular septal defect repair comprised approximately 70%of procedures,while Norwood operations comprised less than one percent of all procedures.Neonatal cardiac procedures were rare in China overall.For procedures in STAT mortality category 1,Chinese centers had lower operative mortality rates,while procedures in categories 3 and 5 mortality is lower in European centers.Operative mortality over the time period decreased from 3.89%to 1.64%for the whole cohort,with a sharper decline in China.This drop coincides with an increase of submitted procedures over this 13-year-period.Conclusion:Chinese centers had higher programmatic volume of congenital heart surgeries,while European centers have a more complex case mix.Palliation for patients with functionally univentricular heart was performed less commonly in China.These comparison of patterns of practice and outcomes demonstrate opportunities for continuing bidirectional transcontinental collaboration and quality improvement.
基金supported by National Institute of Health awards NS118000 and NS128049 (to GG)。
文摘Neurons are energy-demanding cells.Disruptions in energy metabolism can quickly interrupt neuronal function,leading to cell death and neurodegeneration.For instance,ischemia rapidly depletes adenosine triphosphate(ATP)thereby disrupting energy-dependent cellular processes crucial for homeostasis,and axon degeneration is preceded by a collapse of axonal ATP levels.
文摘Axon disconnection in the central nervous system(CNS) usually causes signal transduction failure and severe functional deficits in patients with neurological disorders. Currently, there is no cure for patients with CNS axon injury and they usually suffer from life-long neurological defects(e.g., paralysis, loss of sensory function, and autonomic dysfunction) and life-threatening complications(e.g., autonomic dysreflexia).
基金partially funded by Fondazione Regionale per la Ricerca Biomedica(TRANS-ALS2015-0023)Fondazione“Romeo and Enrica Invernizzi”(to SC and CC)。
文摘Implications for lncRNAs in the central nervous system:Transcriptional dysregulation is a key contributor to the pathogenesis of a wide range of diseases and long non-coding RNAs(lncRNAs)are highly expressed in the nervous system.Indeed,amongst the over 50,000 lncRNAs expressed in the human genome,more than 40%are specifically expressed in the brain where their roles in brain development,neuron functions,maintenance.
基金supported by grants from the National Natural Science Foundation of China(82471937)the Beijing Hospitals Authority Youth Programme(QML20230610).
文摘Background The associations among genetic variants,cardiac magnetic resonance(CMR)features,and prognostic factors of pediatric heart failure(HF)remain unknown.This study aimed to explore the relationship between genetic differences in pediatric HF and CMR findings and their impact on prognosis of HF in children.Methods This retrospective study included children with a first-time diagnosis of HF.All patients underwent echocardiogram,CMR and genetic testing.The cohort was divided into three subgroups according to late gadolinium enhancement(LGE)on CMR:the no LGE subgroup,the subendocardial LGE subgroup,and the nonsubendocardial subgroup.The endpoint was defined as a lack of improvement in HF on echocardiography after more than six months of follow-up.Results Ninety-five children with HF were included,of whom 64 underwent genetic testing and 89 had echocardiograms at follow-up.There were 20 patients in the no LGE group,31 in the subendocardial group,and 44 in the nonsubendocardial group.The most common genotype was MYH7(27.0%).RBM20 and LMNA both presented a dilated phenotype,whereas GTPBP3 uniformly presented a hypertrophic phenotype.TNNI3 showed no LGE on CMR(P=0.003)and maintained a restrictive phenotype.The subendocardial group was more likely to have septal LGE(93.5%,P<0.001).Multivariable regression analysis indicated that a family history of cardiomyopathy,hydropericardium,and right ventricular ejection fraction(RVEF)were independent predictors of the endpoint event(P=0.035,P=0.005 and P=0.032,respectively).Notably,nonsubendocardial LGE was associated with a worse prognosis than no LGE was(P=0.030).Conclusions In pediatric non-ischemic HF patients,subendocardial LGE can be observed.A family history of cardiomyopathy,hydropericardium,and RVEF were predictors of HF with no improvement.The prognosis of HF is associated with the presence of LGE,particularly nonsubendocardial LGE,and less so with the genetic phenotype.
